Introduction
Lymphedema is defined as an excessive lymphatic
fluid accumulation in subcutaneous tissues due to
inability of the lymphatic system to maintain normal
tissue homeostasis.1
It may be classified as primary or secondary. Primary
lymphedema results from congenital abnormality
or dysfunction of the lymphatic vessels.2 Secondary
lymphedema which is more common than the
primary form can develop as a consequence of
destruction or obstruction of lymphatic channels by
other pathological conditions such as infection,
trauma or malignancy. There are three subtypes of
primary lymphedema. Congenital lymphedema
which is detected at birth or during infancy.
Lymphedema praecox which has its onset at the time
of puberty and lymphedema tarda, which usually
occurs after the age of 35 years old.3,4 It is due to an
abnormal lymphangiogenesis in utero, is often
associated with mutation in a gene (VEGFR3,
FOXC2, SOX18, PROX1).5 Because of rarity of this
disease we find academic interest to report the case.
Informed written consent was taken from the parents
after proper explanation.